Synchronous Primary Vulvar Squamous Cell Carcinoma and Ciliated Cell Variant of Endometrioid Adenocarcinoma Arising...

Vulvar carcinoma is an uncommon gynecological malignancy predominantly affecting older women, with vulvar squamous cell carcinoma (VSCC) representing the most common subtype. Endometrial polyps are common benign uterine lesions, although a small proportion may harbor malignant transformation, most often to endometrioid adenocarcinoma. The ciliated cell variant of endometrioid adenocarcinoma (CCVEA) is an exceptionally rare subtype, characterized by neoplastic glands lined by ciliated cells and an indolent clinical course with a favorable prognosis. We report an extremely rare case of CCVEA arising within an endometrial polyp in a 68-year-old postmenopausal woman, who was concurrently diagnosed with VSCC. The patient presented with chronic genital pruritus and a progressively enlarging vulvar lesion. Hysteroscopic evaluation incidentally revealed three endometrial polyps, all of which were removed via polypectomy. Microscopic examination of the polypoidal lesion demonstrated widespread complex endometrial hyperplasia with marked atypia, primarily featuring a lining of ciliated epithelial cells, confirming FIGO (International Federation of Gynecology and Obstetrics) grade IA CCVEA. Concurrent vulvar biopsy showed human papillomavirus (HPV)-independent, well‑differentiated squamous cell carcinoma with prominent keratinization and keratin pearls, staged as FIGO grade II VSCC. The patient underwent radical anterior vulvectomy with distal urethrectomy and meatoplasty, bilateral inguinofemoral lymphadenectomy, exploratory laparotomy, extrafascial hysterectomy with bilateral salpingo‑oophorectomy, and bilateral pelvic lymph node sampling. All lymph nodes were negative for metastasis. At seven months postoperatively, the patient remains disease‑free. This case underscores the risk of synchronous gynecological malignancies and the diagnostic challenges involved in confirming two concurrent primary tumors. It also emphasizes the importance of comprehensive evaluation in postmenopausal women, who may remain asymptomatic despite harboring malignant endometrial polyps. This report presents a rare occurrence of dual primary malignancies in the female genital tract and offers valuable insight that may help guide future treatment protocols.